(S)-beta-Aminoisobutyric acid (BioDeep_00000014452)

 

Secondary id: BioDeep_00000875074, BioDeep_00000897250, BioDeep_00001868745, BioDeep_00001890956

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite PANOMIX_OTCML-2025


代谢物信息卡片


(S)-3-amino-2-Methyl-propanoic acid

  化学式: C4H9NO2 (103.0633)
中文名称: L-3-氨基异丁酸
  谱图信息: 最多检出来源 Homo sapiens(lipidsearch) 30.13%

Reviewed

Last reviewed on 2025-07-03.

Cite this Page

(S)-beta-Aminoisobutyric acid. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/(s)-beta-aminoisobutyric_acid (retrieved 2026-01-13) (BioDeep RN: BioDeep_00000014452). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(CN)C(=O)O
InChI: InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1


描述信息

beta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. The concentration of beta-aminoisobutyric acid is normally low in urine as beta-aminoisobutyric acid is further catabolized by beta-aminoisobutyrate aminotransferases to methylmalonic acid semialdehyde and propionyl-CoA. beta-Aminoisobutyric acid occurs in two isomeric forms and both enantiomers of beta-aminoisobutyric acid can be detected in human urine and plasma. In plasma, the S-enantiomer is the predominant type due to active renal reabsorption. In contrast, urine almost exclusively contains the R-enantiomer of beta-aminoisobutyric acid, which is eliminated both by filtration and tubular secretion. Persistently increased levels of beta-aminoisobutyric acid have been observed in individuals with a deficiency of R (-)-beta-aminoisobutyrate-pyruvate aminotransferase. In addition, transient high levels of beta-aminoisobutyric acid have been observed under a variety of pathological conditions such as lead poisoning, starvation, in total body irradiation, and in a number of malignancies. The S-enantiomer of beta-aminoisobutyric acid is predominantly derived from the catabolism of valine. It has been suggested that altered homeostasis of beta-alanine underlies some of the clinical abnormalities encountered in patients with a dihydropyrimidine dehydrogenase (DPD) deficiency. DPD constitutes the first step of the pyrimidine degradation pathway, in which the pyrimidine bases uracil and thymine are catabolized to beta-alanine and the R-enantiomer of beta-aminoisobutyric acid respectively. In normal individuals with an intact pyrimidine degradation pathway, R-methylmalonic acid semialdehyde can be synthesized directly from the catabolism of thymine. Hence, there might be less cross-over between the valine and thymine pathway, allowing the conversion of S-methylmalonic acid semialdehyde into S-beta-aminoisobutyric acid and the subsequent accumulation of S-beta-aminoisobutyric acid in plasma (PMID: 14705962, 14292857, 14453202).
(S)-b-aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine.

同义名列表

65 个代谢物同义名

(S)-3-amino-2-Methyl-propanoic acid; (2S)-3-amino-2-methylpropanoic acid; (S)-3-Amino-2-methylpropanoic acid; L-3-amino-2-Methylpropanoic acid; L-3-amino-2-Methylpropionic acid; 2S-methyl-3-amino-propanoic acid; (S)-3-amino-2-Methyl-propanoate; (S)-3-Amino-2-methylpropanoate; (+)-beta-Aminoisobutyric acid; (S)-beta-Aminoisobutyric acid; (+)-alpha-Methyl-beta-alanine; (S)-3-Amino-isobutanoic acid; L-3-amino-2-Methylpropanoate; (S)-b-amino-isobutyric acid; S-beta-Aminoisobutyric acid; (S)-3-Aminoisobutanoic acid; L-beta-Aminoisobutyric acid; (S)-3-Amino-isobutyric acid; (+)-b-Aminoisobutyric acid; (S)-3-Aminoisobutyric acid; L-3-Amino-isobutanoic acid; (S)-Β-aminoisobutyric acid; (S)-b-aminoisobutyric acid; L-3-Amino-isobutyric acid; (S)-beta-Aminoisobutyrate; L-b-Aminoisobutyric acid; L-β-aminoisobutyric acid; L-3-Aminoisobutyric acid; (S)-3-Amino-isobutanoate; L-beta-Aminoisobutyrate; S-beta-Aminoisobutyrate; (S)-3-Aminoisobutanoate; (S)-3-Amino-isobutyrate; L-2-Methyl-beta-alanine; L-3-Amino-isobutanoate; (S)-3-Aminoisobutyrate; (+)-a-Methyl-b-alanine; (S)-b-Aminoisobutyrate; (S)-Β-aminoisobutyrate; L-3-Amino-isobutyrate; L-3-Aminoisobutyrate; S-b-Aminoisobutyrate; L-2-Methyl-b-alanine; L-b-Aminoisobutyrate; L-3-Aminoisobutyric acid; (+)-.ALPHA.-METHYL-.BETA.-ALANINE; (+)-ss-Aminoisobutyric acid; (S)-.BETA.-AMINOISOBUTYRIC ACID; (S)-3-AMINO-2-METHYL-PROPIONIC ACID; (S)-3-Amino-2-methylpropanoicacid; (S)-3-AMINO-2-METHYLPROPIONIC ACID; 3-Aminoisobutyric acid, 2S-; Baiba, (S)-; BETA-AMINO ISOBUTYRATE; BIB; H1WR898GX7; L-?-AMINOISOBUTYRIC ACID; L-3-Aminoisobutanoate; L-3-Aminoisobutyric acid, >=97.0% (TLC); L-a-Methyl-ss-alanine; L-Baiba; L-I(2)-Aminoisobutyric acid; S-b-aminoisobutyric acid; S-BAIBA; UNII-H1WR898GX7



数据库引用编号

20 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome()

BioCyc()

PlantCyc()

代谢反应

53 个相关的代谢反应过程信息。

Reactome()

BioCyc()

WikiPathways()

Plant Reactome()

INOH()

PlantCyc()

COVID-19 Disease Map()

PathBank(53)

PharmGKB()

1 个相关的物种来源信息

在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：

  • PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
  • NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
  • Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
  • Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。

点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。

亚细胞结构定位 关联基因列表


文献列表